We acknowledge all staff of Rainbow Children Hospital who lent their support

We acknowledge all staff of Rainbow Children Hospital who lent their support. == Footnotes == Source of Support:Nil Conflict GSK4716 of Interest:No == References ==. test along with smear evidence of hemolysis. He has been treated with intravenous immunoglobulins (IV Igs), packed red cell transfusion and steroids. He had ill-sustained response to conventional therapy; hence, he Mouse monoclonal to BLNK received rituximab, a chimeric monoclonal antibody. == Case Report == A 4-month-old male infant, born to non-consanguineous parents, with uneventful perinatal history and thriving well on breast milk, presented with cough for 10 days and pallor for 2 days. Infant was pale with mild icterus, without any fever and any significant lymphadenopathy or organomegaly. Hemoglobin (Hb), reticulocyte counts were 3.2 g/dL and 6% respectively. Liver function test was normal, but with indirect hyperbilirubinemia (2.5 mg/dL out of 2.9 mg/dL of total bilirubin). Coagulation profile was normal. Direct Coomb’s test (DCT) was strongly positive (4+). High performance liquid chromatography was normal GSK4716 with Hb F being elevated as per age. In urine microscopy, no fresh red blood cell (RBC) but hemolyzed RBCs were seen. There was no Rh or ABO incompatibility setting. Results for anti-nuclear antibodies (ANA) and for retrovirus were negative. Hb level increased to 7.9 g% after packed RBC transfusion (15 mL/kg). However, his repeat Hb 24 h later dropped to 5.8 g% with agglutination in the smear and repeat DCT being again positive (4+). Immunoglobulin G (IgG) auto antibodies were high and diagnosis of AIHA further confirmed. He was started on IV Ig (2 g/kg/total) and on methylprednisolone (10 mg/kg/day). As Hb improved to 9 g% after 4 days, he was discharged with advice to continue oral prednisolone (2 mg/Kg). While tapering steroids, his Hb dropped to 6.7 g%, with repeat DCT being positive and with evidence of hemolysis in smear. A repeat course of IV Igs (1 g/kg) along with stepping up of prednisolone to 1 1 mg/kg/day was given as parents were not consenting for administration of rituximab. There was a transient improvement in Hb to 10 g%, which has dropped later to 7.8 g% on tapering steroids. He was started on rituximab as response to steroids and IV Ig was ill sustained, after obtaining the parent’s consent. Dose was calculated as per the body weight (by taking the standard recommendation of 375 mg/m2once a week for 4 weeks) and 85 mg given once a week. Seven days after the 3rddose, good hematological response (Hb > 10.5 g%, reticulocytes 3%) was observed. Though the patient was covered prophylactically by co-trimoxazole after 3rddose of rituximab he developed severe pneumocystis carinii pneumonia (PCP). The PCP infection was thought to be secondary to GSK4716 immunosuppressive status because of on-going steroid treatment aggravated by rituximab. After rituximab treatment, steroids were GSK4716 tapered over a month and continued at a very low dose (0.2 mg kg/day initially and on alternate day later) for another 2 months as per the hematological picture. Currently, he is off steroids for 6 months from last rituximab dose and maintaining a Hb of 9-10 g%. The DCT is negative. == Discussion == Hemolytic anemia in early infancy is because of either blood group incompatibility or hereditary red cell morphological disorders such as congenital spherocytosis, elliptocytosis or in certain Mediterranean and Asian regions it may be due to red cell enzyme abnormalities, including glucose-6-phosphate dehydrogenase deficiency. It is one of the rare clinical conditions in young infants with significant mortality and morbidity rate up to 10% in children.[4] IV Igs and steroids for immunosuppression are the mainstay of treatment other than support with red cell transfusion where it is possible to get at least near match.